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Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

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Fig. 1.107 Juxtaglomerular cell tumour.<br />

Immunohistochemistry with antibody to renin<br />

shows a diffusely positive reaction in juxtaglomerular<br />

cell tumour.<br />

A<br />

Fig. 1.108 Juxtaglomerular cell tumour. Electron micrograph showing irregular rounded renin-containing<br />

granules (A) and rhomboid crystalline renin granules (B).<br />

B<br />

hypokalemia, although one patient presented<br />

with normal blood pressure<br />

{1044}. Investigation discloses high<br />

plasma renin activity, elevated secondary<br />

hyperaldosteronism and a renal<br />

mass. Hypertension and hypokalemia<br />

resolve after surgery.<br />

Macroscopy and histopathology<br />

JGCT is solid, well-circumscribed and<br />

yellow-tan. The tumour is usually smaller<br />

than 3 cm in diameter but cases ranging<br />

from 2 mm {1097} to 9 cm {1413} have<br />

been reported. JGCT is histologically<br />

made of sheets of polygonal or spindled<br />

tumour cells with central round regular<br />

nuclei, distinct cell borders and abundant<br />

granular eosinophilic cytoplasm<br />

staining for the Bowie stain, PAS and<br />

toluidine blue. Typically, tumours present<br />

with a complex vascular hemangiopericytic<br />

pattern. Mast cells and<br />

thick-walled hyalinized blood vessels<br />

are common and, in about one-half of<br />

reported cases, prominent tubular elements<br />

either neoplastic or entrapped<br />

are also present. Rarely, JGCT may be<br />

largely papillary {2602}. Tumour cells<br />

are immunoreactive for renin, actin,<br />

Fig. 1.109 Juxtaglomerular cell tumour. Renin expression in some cells.<br />

vimentin and CD34 {1638}. Ultrastructural<br />

features include abundant<br />

rough endoplasmic reticulum, a well<br />

developed Golgi apparatus and numerous<br />

peripherally located sharply angulated<br />

rhomboid renin protogranules. A<br />

variable number of round electrondense<br />

mature renin-like granules are<br />

also found.<br />

Juxtaglomerular cell tumour<br />

73

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